[Male pseudohermaphroditism. State and present problems (author's transl)].

The authors describe the male phenotypic sexual development in the fetus: transformation of the undifferentiated gonad into testis under the HY antigen influence; testicular secretion of testosterone (which allows the development of the wolffian ducts derivatives) and of MIF (which inhibits the development of the mullerian duct; masculinization of the external genitalia under the influence of dihydrotestosterone. They outline the three abnormalities which may lead to a male pseudohermaphroditism: disorders of testicular differentiation, defects of testicular function and androgen insensitiveness at the target areas. They consider the diagnostic steps and the therapeutic management especially in relation to the choice of the sex and the risk of gonadoblastoma.